What is the autoimmune disease characterized by circulating IgG autoantibodies against two major podocyte antigens?
PRIMARY MEMBRANOUS NEPHROPATHY (PMN)
What are the 2 major podocyte antigens in Primary MN?
- PLA2R1 (70%)
- THSDA (5-10%)
What are the 2 new classification for MPGN
- IMMUNE COMPLEX-MEDIATED
- COMPLEMENT- MEDIATED OR C3 GLOMERULOPATHY
New classification of MPGN is based on:
What type of MPGN, seen in IF where both immunoglobulin and complement are deposited
Immune complex-mediated MPGN
What type of MPGN with predominant C3 staining by IF?
Complement-mediated MPGN or C3 glomerulopathy
An autoimmune disease which results from autoantibodies targeting DNAJB9 (a putative autoantigen)
Patients with immunotactoid glomerulonephritis should be evaluated for the underlying 3 disorders:
- Monoclonal gammopathy
- Lymphoproliferative disorders
Autoantibodies in sporadic anti-GBM disease are directed against NC1 domain of what chain of type IV collagen?
What antibody in ANCA-associated vasculitis, which when present lung involvement, or upper respiratory tract involvement are more likely to relapse?
ANTI-PROTEINASE-3 (PR3) ANTIBODIES
Anti-GBM alloantibodies cause posttransplantation nephritis in X-linked Alport syndrome are directed against the NC1 domain of what chain of the type IV collagen?
IgA vasculitis was previously called as?
GPA (Granulomatosis with polyangiitis) was previously called?
EGPA (Eosinophilic granulomatosis with polyangiitis) was previously called as?
What are the 4 glomerular disease with Nephrotic Features?
- FIBRILLARY GN
What glomerular disease manifest purely with Nephrotic features?
Loss of charge selectivity is the cause of proteinuria in what type of GN?
Loss of size selectivity is the cause of proteinuria in what type of GN
What is the strong evidence for glomerular bleeding?
What are the 4 differential diagnosis of glomerular hematuria without proteinuria, renal insufficiency, or red blood cell casts?
- IgA NEPHROPATHY
- HEREDITARY NEPHRITIS
- NORMAL GLOMERULI
What is the protein excretion rate in Nephrotic syndrome?
Refers to the presence of lipid in the tubular epithelial cells and urine
MCD was previously called as?
What the the 6 factors that are increased in Nephrotic syndrome?
2, 5, 7, 8, 10, 13
What are the 3 factors that are decreased in Nephrotic syndrome?
9, 11, 12
The most consistent tubular lesion of increased protein & lipid resorption droplets in TECs is seen in what type of GN?
What is the most frequent positive IF finding in MCD?
Low-level mesangial staining for IgM & C3
What is the pathologic sine qua non of MCD in EM?
EFFACEMENT OF FOOT PROCESSES
What are the LM, IF, EM findings in MCD?
LM: no glomerular lesions
*focal segmental mesangial prominence (not >3-4cells)
IF: no staining
*low level staining for IgM,C3
EM: effacement of foot processes
In MCD, there is abnormal regulation of this cell subset:
What NSAID has been associated with MCD?
What type of lymphoma is associated with MCD?
CLASSIC HODGKIN LYMPHOMA (NODULAR SCLEROSIS TYPE)
What is dose of prednisone for the treatment of MCD?
Not to exceed 80mg/day
What is considered a response to therapy in MCD?
No proteinuria by dipstick for at least 3 days
In adult patients with MCD, a response to corticosteroid treatment may take up to how many weeks?
In MCD, how long should treatment with corticosteroids continued after complete remission of proteinuria?
What is the dose of oral cyclophosphamide in the treatment of MCD?
What is an alternative to cyclophosphamide therapy for relapsing or steroid-dependent MCD?
What is the duration of treatment with cyclosporine for relapsing/steroid-dependent MCD?
What are the 2 types of CNI?
B lymphocyte-depleting agent?
What is the usual treatment regimen for MCD?
- Prednisone (induction of remission)
Response: 15 weeks
Continued after remission: 6 more weeks
- Cyclophosphamide (maintenance)
What is a differential diagnosis in steroid-resistant MCD?
What are the 2 treatment options for steroid-resistant MCD?
What are the 10 glomerular disease that cause Nephrotic syndrome & GN?
- C1q NEPHROPATHY
- C3 GLOMERULOPATHY
- ACUTE PSGN
- FIBRILLARY GN
- IMMUNOTACTOID GN